Œdème angioneurotique héréditaire : à propos de deux cas

Summary We report two cases of hereditary angioedema (HAE) in two members of the same family. Angioedema is caused by a deficiency of C1 inhibitor. It shows itself by recurrent, non-inflammatory and non-pruritic episodes of subcutaneous and/or submucosal edema of extremities and face or by pseudo-occlusive abdominal attacks, spontaneously reversible. The exploration of the classical complement pathway during or subsequent to the crisis, fundamental for the diagnosis, shows a decrease of CH50 and C4 complement. The C3 is normal. Weight filling of C1 inhibitor, collapsed in such cases, signs the diagnosis. The occurrence of angioedema in a mother and her daughter suggests a genetic origin; the hereditary form of the disease is most frequently described in the literature. However, in our case, antinuclear antibodies (ANA) detected at a relatively high titer, in one case, and the association of angioedema with rheumatoid arthritis, in the other case, raises the problem of an acquired associated component on this autoimmune background.